Reader response: Clinical characteristics, risk factors, and outcomes of POEMS syndrome: A longitudinal cohort study
ChiaraBriani, Neurologist, Department of Neurosciences, University of Padova (Padova, Italy)
RenzoManara, Neuroradiologist, Neuroradiology, University of Padova (Padova, Italy)
AnnalisaAngelini, Pathologist, Cardiovascular Pathology, University of Padova (Padova, Italy)
Submitted July 21, 2020
While congratulating Keddie et al.1 for the informative article, we would like to specify the reasons why we hypothesized a role of angiogenic factors in the meningeal thickening in POEMS syndrome in our manuscript to which the authors referred.2
As Keddie et al., we also did not find correlation between meningeal thickening and disease duration or Vascular endothelial growth factor (VEGF) serum level in POEMS patients. However, in our study, histopathologic findings in meningeal specimens of POEMS patients showed increased vessel density and thickness, different from meninges from healthy subjects or rheumatologic patients. No inflammatory cell infiltration was detected in POEMS meninges. Moreover, both single and double immunostaining showed strong expression of VEGFR-2 in smooth muscle cells, endothelial and meningothelial cells in meninges from POEMS patients. VEGF and VEGFR2 were instead absent in healthy controls.2
Further support to a role of angiogenic factors in pachymeningeal remodelling comes from the MRI changes in POEMS patients after therapy with lenalidomide, known to have anti-angiogenic activity.3
In conclusion, while acknowledging that neither the histopathologic data nor the pachymeningeal MRI improvement after anti-angiogenic therapy are a direct proof of a VEGF role in pachymeningeal involvement in POEMS patients; however, we believe that our data strongly support this hypothesis.
Disclosure
The authors report no relevant disclosures. Contact journal@neurology.org for full disclosures.
References
Keddie S, Foldes D, Caimari F, et al. Clinical characteristics, risk factors, and outcomes of POEMS syndrome: A longitudinal cohort study. Neurology. 2020;95:e268–e279.
Briani C, Fedrigo M, Manara R, et al. Pachymeningeal involvement in POEMS syndrome: MRI and histopathological study. J Neurol Neurosurg Psychiatry 2012;83:33–37.
Briani C, Manara R, Lessi F, et al. Pachymeningeal involvement in POEMS syndrome: dramatic cerebral MRI improvement after lenalidomide therapy. Am J Hematol. 2012;87:539–541.
While congratulating Keddie et al.1 for the informative article, we would like to specify the reasons why we hypothesized a role of angiogenic factors in the meningeal thickening in POEMS syndrome in our manuscript to which the authors referred.2
As Keddie et al., we also did not find correlation between meningeal thickening and disease duration or Vascular endothelial growth factor (VEGF) serum level in POEMS patients. However, in our study, histopathologic findings in meningeal specimens of POEMS patients showed increased vessel density and thickness, different from meninges from healthy subjects or rheumatologic patients. No inflammatory cell infiltration was detected in POEMS meninges. Moreover, both single and double immunostaining showed strong expression of VEGFR-2 in smooth muscle cells, endothelial and meningothelial cells in meninges from POEMS patients. VEGF and VEGFR2 were instead absent in healthy controls.2
Further support to a role of angiogenic factors in pachymeningeal remodelling comes from the MRI changes in POEMS patients after therapy with lenalidomide, known to have anti-angiogenic activity.3
In conclusion, while acknowledging that neither the histopathologic data nor the pachymeningeal MRI improvement after anti-angiogenic therapy are a direct proof of a VEGF role in pachymeningeal involvement in POEMS patients; however, we believe that our data strongly support this hypothesis.
Disclosure
The authors report no relevant disclosures. Contact journal@neurology.org for full disclosures.
References